Scleroderma is a disease of the body's connective tissue. Scleroderma means 'hard skin'. The most common symptom is a thickening and hardening of the skin, particularly of the hands and face.
The millions of cells that make up the human body are held together by meshes of connective tissue that support and hold together our joints, muscles, internal organs and skin. The connective tissues of people with scleroderma have too much collagen, causing hardening and tightening of this connective tissue.
Many different areas of the body can be affected by scleroderma, and symptoms vary greatly from person to person.
Anyone can develop scleroderma, but it is more common between the ages of 30 and 60, and three times more likely in females. There are estimated to be over 5,000 Australians living with scleroderma.
Scleroderma is not contagious. The condition can be mild, moderate or severe. There is no cure, but it is possible to successfully manage the symptoms.
Types of scleroderma
There are two broad classifications of scleroderma based on the amount of skin and other organs affected. These are:
- Localised scleroderma affects the skin. There are two types of localised scleroderma, morphea or linear. Both have distinctive signs and symptoms. Localised scleroderma can sometimes restrict normal joint movement, due to hardening of the skin over the joint. The cause of localised scleroderma is unknown.
- Diffuse scleroderma (also called systemic scleroderma or systemic sclerosis) involves a more widespread hardening of the skin. Internal organs are also affected. Systemic sclerosis is an autoimmune disease, meaning that the hard skin and involvement of internal organs arise as a consequence of immune system overactivity such that it attacks the body’s own tissues.
Symptoms of scleroderma
The symptoms will vary from person to person, and will depend on the type of scleroderma, and the organ involvement.
Symptoms can include:
- thickening and hardening of the skin
- Raynaud's phenomenon – a condition which affects the blood flow to the extremities, most often fingers and toes. It is caused by a sudden constriction of the blood vessels
- stiffness and pain in the muscles or joints (or both)
- indigestion, heartburn, diarrhoea, constipation.
Causes of scleroderma
The cause of scleroderma is not known. Possible causes may include an overactive immune system, genetics and environmental triggers, although none of these factors has been proven.
Diagnosis of scleroderma
A dermatologist can usually diagnose scleroderma with a visual examination of the skin (confirmed with a skin biopsy). You may also require a range of medical tests, including physical examinations, blood tests and tissue biopsies to reach a diagnosis.
Treatment of scleroderma
There is no cure for scleroderma. Treatment aims to ease the symptoms and will be dependent on the person's symptoms. Steroid creams applied to the skin may be prescribed for localised scleroderma. A range of other anti-inflammatory medications may also be prescribed. Specialised medications are also available for scleroderma-associated lung disease.
A person with scleroderma should be monitored by a dermatologist or rheumatologist. Other healthcare providers such as immunologists, respiratory physicians and gastroenterologists may take part in ongoing treatment.
Management of scleroderma
Managing the disease depends on its severity, but may include:
- medication – to decrease the symptoms of Raynaud's phenomenon or treat other affected organs such as the gastrointestinal tract, lungs or kidneys
- lifestyle changes – avoid exposure to cold temperatures, dress warmly and don't smoke. These measures help manage Raynaud's phenomenon
- gentle, regular exercise – to counteract the effects of joint immobilisation and improve overall health
- stress management – to help you cope with the demands and challenges of their disease
- aids and equipment – the effects of scleroderma may make some actions difficult, for example, using door handles or getting dressed. Changing your home or work environment and using aids and equipment can help. An occupational therapist can provide advice.
Where to get help
- Your doctor
- Scleroderma Foundation of Victoria Tel. (03) 9288 3651
- Arthritis Victoria Tel. (03) 8531 8000 or 1800 011 041
Things to remember
- Scleroderma is a chronic condition that affects the connective tissue.
- The disorder can be mild, moderate or severe, depending on how much skin and connective tissue is affected.
- Treatment aims to treat the symptoms, and will vary from person to person.
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